US2025145642A1PendingUtilityA1

Bicyclic heteroarenes and methods of their use

60
Assignee: KINETA INCPriority: Dec 8, 2021Filed: Dec 8, 2022Published: May 8, 2025
Est. expiryDec 8, 2041(~15.4 yrs left)· nominal 20-yr term from priority
A61P 25/28A61P 25/00C07D 498/04C07D 487/04C07D 473/32A61K 31/5383A61K 31/5377C07D 471/04C07D 519/00
60
PatentIndex Score
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Claims

Abstract

Disclosed are compounds useful in the treatment of neurological disorders. The compounds described herein, alone or in combination with other pharmaceutically active agents, can be used for treating or preventing neurological diseases, including frontotemporal dementia, chronic traumatic encephalopathy, Alzheimer's disease, limbic-predominant age-related TDP-43 encephalopathy (LATE), or frontotemporal lobar degeneration.

Claims

exact text as granted — not AI-modified
1 . A compound of formula (I): 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein 
            is a single bond, X 1  is (C(R A ) 2 ) m  or —OC(R A ) 2 —R X , and X 2  is C(R A ) 2  or CO; or   is a double bond, and each of X 1  and X 2  is independently CR A  or N, wherein R X  is a bond to X 2 ; 
         R 1  is —(L) n —R B ; halo, cyano, hydrogen, optionally substituted C 1-6  alkoxy, optionally substituted C 1-9  heterocyclyl comprising at least one endocyclic oxygen, optionally substituted C 1 -C 6  alkyl, optionally substituted piperazin-1-yl, optionally substituted pyrrolidine-3-yl, pyrimidinyl optionally substituted with cyclopropyl or optionally substituted C 6 -C 10  aryl, optionally substituted pyridazinyl, optionally substituted oxazolyl, pyrid-2-on-1-yl, optionally substituted isoindolinyl, unsubstituted pyridin-4-yl, unsubstituted pyridin-2-yl, optionally substituted furan-3-yl, unsubstituted pyridin-3-yl, or optionally substituted pyrazol-1-yl; 
         R 2  is optionally substituted C 1 -C 6  alkyl, optionally substituted C 6 -C 10  aryl, optionally substituted piperidin-4-yl, optionally substituted tetrahydropyran-4-yl, optionally substituted pyrimidin-5-yl, optionally substituted pyrimidin-4-yl, optionally substituted pyridine-3-yl, optionally substituted pyridazin-4-yl, optionally substituted pyrazol-1-yl, optionally substituted pyrazol-4-yl, optionally substituted pyrazol-3-yl, optionally substituted pyridine-2-yl, optionally substituted triazolyl, optionally substituted benzodioxol-2-yl, optionally substituted benzodioxan-2-yl, optionally substituted C 6 -C 10  aryl C 1 -C 10  alkyl, or optionally substituted acyl; 
         R 3  is a group of the following structure: 
       
       
         
           
           
               
               
           
         
         each R A  is independently H, optionally substituted C 1-6  alkyl, or optionally substituted C 6 -C 10  aryl, or two R A , together with the atom to which they are attached, combine to form oxo; wherein when two R A , together with the atom to which they are attached, combine to form oxo, then   is a single bond; 
         R B  is optionally substituted C 6-10  aryl, optionally substituted C 1 -C 9  heteroaryl, optionally substituted C 3-8  cycloalkyl, —N═CH—R D , or optionally substituted C 1 -C 9  heterocyclyl, optionally substituted C 2 -C 9  heteroaryl C 1 -C 6  alkyl, optionally substituted C 2 -C 9  heterocyclyl C 1-6  alkyl; 
         R C  is H or optionally substituted C 1 -C 6  alkyl; 
         R D  is optionally substituted C 6 -C 10  aryl; or 
         each L is independently optionally substituted C 1-6  alkylene, optionally substituted C 1 -C 6  heteroalkylene, optionally substituted C 3 -C 8  cycloalkylene, optionally substituted C 2 -C 6  alkynylene, O, or NR C —; 
         n is 1, 2, or 3; and 
         m is 0, 1, or 2. 
       
     
     
         2 . The compound of  claim 1 , wherein   is a single bond. 
     
     
         3 . The compound of  claim 1 or 2 , wherein X 1  is (C(R A ) 2 ) m . 
     
     
         4 . The compound of  claim 3 , wherein m is 1. 
     
     
         5 . The compound of any one of  claims 1 to 4 , wherein X 2  is C(R A ) 2 . 
     
     
         6 . The compound of any one of  claims 1 to 5 , wherein each R A  is hydrogen. 
     
     
         7 . The compound of  claim 1 , wherein the compound is of formula (1a): 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         8 . The compound of  claim 1 , wherein the compound is of formula 1a′): 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         9 . The compound of  claim 1 , wherein the compound is of formula (1b): 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         10 . The compound of  claim 1 , wherein the compound is of formula (1c): 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         11 . The compound of  claim 1 , wherein the compound is of formula (1d): 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         12 . The compound of  claim 1 , wherein the compound is of formula (1e): 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         13 . The compound of any one of  claims 1 to 12 , wherein R 1  is —O—(L) (n-1) —R B . 
     
     
         14 . The compound of any one of  claims 1 to 13 , wherein n is 2. 
     
     
         15 . The compound of any one of  claims 1 to 13 , wherein n is 1. 
     
     
         16 . The compound of any one of  claims 1 to 15 , wherein at least one L is optionally substituted C 1-6  alkylene. 
     
     
         17 . The compound of  claim 16 , wherein L is methylene. 
     
     
         18 . The compound of  claim 16 , wherein L is ethylene. 
     
     
         19 . The compound of any one of  claims 1 to 18 , wherein R B  is optionally substituted C 1-9  heterocyclyl. 
     
     
         20 . The compound of any one of  claims 1 to 18 , wherein R B  is optionally substituted C 1-9  heteroaryl. 
     
     
         21 . The compound of any one of  claims 1 to 18 , wherein R B  is optionally substituted C 1-6  alkyl. 
     
     
         22 . The compound of any one of  claims 1 to 21 , wherein R A  is optionally substituted C 1-6  alkyl. 
     
     
         23 . The compound of  claim 22 , wherein R A  is methyl. 
     
     
         24 . The compound of any one of  claims 1 to 21 , wherein R 1  is optionally substituted C 1 -C 6  alkyl. 
     
     
         25 . The compound of  claim 24 , wherein R 1  is optionally substituted C 1-6  alkyl. 
     
     
         26 . The compounds of  claims 24 or 25 , wherein R 1  is substituted with hydroxyl. 
     
     
         27 . The compound of any one of  claims 1 to 21 , wherein R 1  is optionally substituted piperazin-1-yl. 
     
     
         28 . The compound of  claim 27 , wherein R 1  is substituted with methyl. 
     
     
         29 . The compound of any one of  claims 1 to 28 , wherein R 2  is optionally substituted C 6 -C 10  aryl C 1 -C 6  alkyl. 
     
     
         30 . The compound of  claim 29 , wherein R 2  is optionally substituted C 6 -C 10  aryl C 1 -C 2  alkyl. 
     
     
         31 . The compound of  claim 29 or 30 , wherein R 2  is substituted with oxo. 
     
     
         32 . The compound of any one of  claims 1 to 28 , wherein R 2  is optionally substituted C 1 -C 6  alkyl. 
     
     
         33 . The compound of  claim 32 , wherein R 2  is optionally substituted C 4  alkyl. 
     
     
         34 . The compound of  claim 32 or 33 , wherein R 2  is substituted with hydroxyl. 
     
     
         35 . The compound of any one of  claims 1 to 28 , wherein R 2  is an optionally substituted C 6 -C 10  aryl. 
     
     
         36 . The compound of  claim 35 , wherein R 2  is optionally substituted phenyl. 
     
     
         37 . The compound of  claim 35 or 36 , wherein R 2  is substituted with fluoro, cyano, or methoxy. 
     
     
         38 . The compound of any one of  claims 1 to 12 or 29 to 37 , wherein R 1  is: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
       methyl, or methoxy. 
     
     
         39 . The compound of  claim 38 , wherein R 1  is 
       
         
           
           
               
               
           
         
       
       or methoxy. 
     
     
         40 . The compound of any one of  claims 1 to 28 or 38 , wherein R 2  is: 
       
         
           
           
               
               
           
         
         
           
           
               
               
           
         
       
     
     
         41 . The compound of  claim 40 , wherein R 2  is: 
       
         
           
           
               
               
           
         
       
     
     
         42 . The compound of any one of  claims 1 to 41 , wherein R 3  is: 
       
         
           
           
               
               
           
         
       
     
     
         43 . The compound of  claim 1 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein R 2  is optionally substituted pyrimidin-3-yl or optionally substituted pyrimidin-4-yl; and 
         R 4  is hydrogen or optionally substituted C 6 -C 10  aryl. 
       
     
     
         44 . The compound of  claim 43 , wherein R 4  is hydrogen. 
     
     
         45 . The compound of  claim 44 , wherein R 4  is optionally substituted C 6 -C 10  aryl. 
     
     
         46 . The compound of  claim 45 , wherein R 4  is phenyl. 
     
     
         47 . The compound of  claim 45 or 46 , wherein R 4  is substituted with fluoro. 
     
     
         48 . The compound of  claim 45 or 46 , wherein R 4  is substituted with methoxy. 
     
     
         49 . The compound of any one of  claims 43 to 48 , wherein R 2  is optionally substituted pyridin-3-yl. 
     
     
         50 . The compound of any one of  claims 43 to 48 , wherein R 2  is pyridin-4-yl. 
     
     
         51 . The compound of  claim 1 or 29 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein R 5  is hydrogen or optionally substituted C 6 -C 10  aryl; and 
         R 2  is optionally substituted triazolyl, optionally substituted pyrazol-4-yl, optionally substituted pyrazol-3-yl, optionally substituted pyrimidin-4-yl, or optionally substituted C 6 -C 10  aryl C 1-6  alkyl. 
       
     
     
         52 . The compound of  claim 51 , wherein R 5  is hydrogen. 
     
     
         53 . The compound of  claim 51 , wherein R 5  is phenyl. 
     
     
         54 . The compound of any one of  claims 51 to 53 , wherein R 2  is substituted with phenyl. 
     
     
         55 . The compound of any one of  claims 51 to 54 , wherein R 2  is 
       
         
           
           
               
               
           
         
       
     
     
         56 . The compound of  claim 51 , wherein R 2  is 
       
         
           
           
               
               
           
         
       
     
     
         57 . The compound of  claim 1 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein R 2  is optionally substituted pyridin-3-yl. 
       
     
     
         58 . The compound of  claim 1 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein R 2  is optionally substituted C 6 -C 10  aryl; or optionally substituted pyridzin-4-yl. 
       
     
     
         59 . The compound of  claim 58 , wherein R 2  is 3-fluoro-phenyl. 
     
     
         60 . The compound of  claim 58 , wherein R 2  is pyridazin-4-yl. 
     
     
         61 . The compound of  claim 1 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein R 2  is optionally substituted pyridin-3-yl. 
       
     
     
         62 . The compound of  claim 1 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein L is optionally substituted C 3 -C 8  cycloalkylene or C 2 -C 6  alkynylene; and 
         R B  is optionally substituted C 6 -C 10  aryl. 
       
     
     
         63 . The compound of  claim 62 , wherein L is 
       
         
           
           
               
               
           
         
       
     
     
         64 . The compound of  claim 63 , wherein L is 
       
         
           
           
               
               
           
         
       
     
     
         65 . The compound of any one of  claims 62 to 64 , wherein R B  is 3-methoxy-phenyl. 
     
     
         66 . The compound of  claim 1 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein L—R B  is —NHN═CHR D ; 
         R 2  is optionally substituted pyridine-3-yl; and 
         R D  is optionally substituted C 6 -C 10  aryl. 
       
     
     
         67 . The compound of  claim 66 , wherein R D  is 3-methyl-phenyl. 
     
     
         68 . The compound of  claim 66 or 67 , wherein R 2  is pyridin-3-yl. 
     
     
         69 . The compound of any one of  claims 66 to 68 , wherein the compound has the structure 
       
         
           
           
               
               
           
         
       
       or a pharmaceutically acceptable salt thereof. 
     
     
         70 . The compound of  claim 1 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein R 2  is optionally substituted pyridine-3-yl; and 
         R 6  is optionally substituted C 6 -C 10  aryl. 
       
     
     
         71 . The compound of  claim 70 , wherein Re is 3-methyl-phenyl or phenyl. 
     
     
         72 . A compound, or pharmaceutically acceptable salt thereof, having the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein X 2  is N or CH; 
         R A  is optionally substituted C 2 -C 9  heteroaryl; or optionally substituted C 6 -C 10  aryl; 
         R 1  is optionally substituted C 2 -C 9  heteroaryl or —O—R 7 ; 
         R 2  is hydrogen or optionally substituted C 1 -C 6  alkyl; and 
         R 7  is optionally substituted C 2 -C 9  heteroaryl C 1-6  alkyl. 
       
     
     
         73 . The compound of  claim 72 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         74 . The compound of  claim 72 or 73 , wherein R 2  is 2-hydroxy-ethyl. 
     
     
         75 . The compound of  claim 72 or 73 , wherein R 2  is hydrogen. 
     
     
         76 . The compound of  claim 72 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         77 . The compound of  claim 72 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         78 . The compound of any one of  claims 72 to 77 , wherein R A  is optionally substituted phenyl. 
     
     
         79 . The compound of any one of  claims 72 to 77 , wherein R A  is optionally substituted pyridine-2-yl, optionally substituted pyrimidin-4-yl, optionally substituted pyrimidin-2-yl, optionally substituted pyrazol-4-yl, optionally substituted pyridine-3-yl, optionally substituted pyrazol-4-yl, optionally substituted 7-aza-5,6,7,8-tetrahydroindolizin-1-yl, optionally substituted pyridazin-3-yl, or optionally substituted pyridine-4-yl. 
     
     
         80 . The compound of any one of  claims 72 to 77 , wherein R A  is optionally substituted C 2 -C 9  heteroaryl or C 6 -C 10  aryl substituted with optionally substituted C 2 -C 9  heteroaryl. 
     
     
         81 . The compound of any one of  claims 72 to 80 , wherein R A  is substituted with cyclopropyl, methyl, methoxy or 
       
         
           
           
               
               
           
         
       
     
     
         82 . The compound of  claim 80 , wherein R A  is phenyl substituted with pyrazol-1-yl. 
     
     
         83 . The compound of any one of  claims 72 to 77 , wherein R A  is 
       
         
           
           
               
               
           
         
       
     
     
         84 . The compound of any one of  claims 72 to 83 , wherein R 1  is pyridine-3-yl, pyridine-4-yl, or 
       
         
           
           
               
               
           
         
       
     
     
         85 . The compound of  claim 76 , wherein the compound has the structure 
       
         
           
           
               
               
           
         
       
       or a pharmaceutically acceptable salt thereof. 
     
     
         86 . The compound of  claim 77 , wherein the compound has the structure: 
       
         
           
           
               
               
           
         
       
       or a pharmaceutically acceptable salt thereof. 
     
     
         87 . A compound of the following structure: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                   # 
                   Compound 
                 
                     
                     
                 
                     
                 
                 
                 
                 
               
                     
                   1 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   2 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
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                   20 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
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                   25 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   26 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   27 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   28 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   29 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   30 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   31 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
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                   33 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   34 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   35 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
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                   38 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
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                   40 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   41 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   42 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
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                   44 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   45 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
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                   50 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   53 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
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                   64 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   65 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
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                   111 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   112 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   113 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   114 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   115 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   116 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   117 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   118 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   119 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   120 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   121 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   126 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   127 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   128 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   129 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   130 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   132 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   135 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
                     
                   151 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         88 . A compound of the following structure: 
       
         
           
                 
                 
               
                     
                 
                   # 
                   Compound 
                 
                     
                 
                     
                 
                 
                 
               
                   51 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   52 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   67 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   68 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   69 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   70 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   71 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   72 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   73 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   74 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   75 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   82 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   83 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   84 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   85 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   93 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   94 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   95 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   96 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   97 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   122 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   123 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   124 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   125 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   131 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   133 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   134 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   136 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   137 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   138 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   139 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   140 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   141 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   142 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   143 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   144 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   145 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   146 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   147 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   148 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   149 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
                   150 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                 
                     
                 
             
                
                
                
               
               
                
               
            
             
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
     
     
         89 . A pharmaceutical composition comprising the compound of any one of  claims 1 to 88 , or a pharmaceutically acceptable salt thereof, pharmaceutically acceptable excipient. 
     
     
         90 . A method of treating a neurological disorder in a subject in need thereof, the method comprising administering to the subject an effective amount of the compound of any one of  claims 1 to 88 , or a pharmaceutically acceptable salt thereof or the pharmaceutical composition of  claim 89 . 
     
     
         91 . The method of  claim 90 , wherein the neurological disorder is FTLD-TDP, chronic traumatic encephalopathy, ALS, Alzheimer's disease, LATE, or frontotemporal lobar degeneration. 
     
     
         92 . The method of  claim 91 , wherein the neurological disorder is ALS. 
     
     
         93 . A method of inhibiting toxicity in a cell related to a protein, the method comprising contacting the cell with the compound of any one of  claims 1 to 88  or a pharmaceutically acceptable salt thereof. 
     
     
         94 . The method of  claim 93 , wherein the toxicity is TDP-43-related toxicity. 
     
     
         95 . The method of  claim 93 , wherein the toxicity is C 9 orf72-related toxicity. 
     
     
         96 . A method of inhibiting PIKfyve in a cell expressing PIKfyve protein, the method comprising contacting the cell with the compound of any one of  claims 1 to 88  or a pharmaceutically acceptable salt thereof. 
     
     
         97 . The method of any one of  claims 93 to 96 , wherein the cell is a mammalian neural cell. 
     
     
         98 . The method of any one of  claims 93 to 97 , wherein the cell is in a subject. 
     
     
         99 . The method of  claim 98 , wherein the subject suffers from a neurological disorder. 
     
     
         100 . A method of treating a TDP-43-associated disorder in a subject, the method comprising administering to the subject in need thereof an effective amount of the compound of formula (14): 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof, 
         wherein 
            is a single bond, X 1  is (C(R A ) 2 ) m  or —OC(R A ) 2 —R X , and X 2  is C(R A ) 2  or CO; or   is a double bond, and each of X 1  and X 2  is independently CR A  or N, wherein R X  is a bond to X 2 ; 
         R 1  is —(L) n —R B , hydrogen, halogen, cyano, optionally substituted C 1-6  alkyl, optionally substituted C 1-6  heteroalkyl, optionally substituted C 1-6  alkoxy, optionally substituted C 6-10  aryl, optionally substituted C 1-9  heterocyclyl, or optionally substituted C 1-9  heteroaryl; 
         R 2  is hydrogen, optionally substituted C 1-6  alkyl, optionally substituted C 6-10  aryl, optionally substituted C 1-9  heterocyclyl, optionally substituted C 1-9  heteroaryl, or optionally substituted C 6 -C 10  aryl C 1 -C 6  alkyl; 
         R 3  is a group of the following structure: 
       
       
         
           
           
               
               
           
         
         each R A  is independently H, optionally substituted C 1-6  alkyl, optionally substituted C 6-10  aryl, or two geminal R A  groups, together with the atom to which they are attached, combine to form oxo; 
         R B  is optionally substituted C 6-10  aryl, optionally substituted C 1-9  heteroaryl, optionally substituted C 3-8  cycloalkyl, —N═CH—R D , or optionally substituted C 1-9  heterocyclyl, optionally substituted C 2 -C 9  heteroaryl C 1-6  alkyl, optionally substituted C 2 -C 9  heterocyclyl C 1-6  alkyl; 
         R C  is H or optionally substituted C 1 -C 6  alkyl; 
         R D  is optionally substituted C 6 -C 10  aryl; 
         each L is independently optionally substituted alkylene, optionally substituted C 1 -C 6  heteroalkylene, optionally substituted C 3 -C 8  cycloalkylene, optionally substituted C 2 -C 6  alkynylene, O, or NR C ; and 
         n is 1, 2, or 3; and 
         m is 0, 1, or 2.

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